Epidemiologic trends and outcomes of primary vitreoretinal lymphoma in the U.S., 2000-2021.

PURPOSE: Primary vitreoretinal lymphoma (PVRL) is a rare and aggressive tumour. Most existing evidence comes from low-powered, historical cohorts. In addition, its incidence is usually indirectly estimated from primary central nervous system lymphoma and is limited to pre-2010 cohorts. Herein, we analyse recent trends in the incidence, sociodemographic characteristics, and survival outcomes of PVRL using U.S. national databases. METHODS: Patients diagnosed with PVRL between 2000 and 2021 were identified using the 2 national population databases, National Program of Cancer Registries (NPCR) and Surveillance, Epidemiology and End Results Research (SEER) Plus. Incidence, sociodemographic characteristics, and survival outcomes were calculated. Predictors for survival were analysed using restricted mean survival time analysis. RESULTS: NPCR and SEER Plus had a total of 267 and 76 patients, respectively. The age-adjusted incidence was 0.004 cases per 100 000. Using the SEER database, we found there was a significant increase in the age-adjusted incidence rates between 2000 and 2021, with an annual percentage change of 5.10. In addition, there was a significant increase in patients aged older than 75 years over time. Patients were more commonly White, non-Hispanic, and 70-74 years of age. Black race was associated with worse survival (P = 0.0001). Type of treatment was not associated with survival (P > 0.05). CONCLUSIONS: PVRL is underrepresented in national cancer databases, possibly as the result of misdiagnosis or coding errors. However, its incidence continues to increase in the U.S., with a significantly older population of patients diagnosed in 2021 compared with 2000. Significant racial disparities exist in the survival of PVRL.